Idiopathic Pleuroparenchymal Fibroelastosis

Amitani and colleagues first described a unique pattern of upper lobe–predominant, idiopathic pulmonary fibrosis (IPF) in 13 patients in the Japanese literature in 1992. The currently preferred term in the English-language literature, idiopathic pleuroparenchymal fibroelastosis (iPPFE), was coined by Frankel and colleagues in 2004 in a case series of five patients presenting with unique clinical and radiographic findings distinct from any of the previously characterized idiopathic interstitial pneumonias (IIPs). Since that time, a number of cases of pleuroparenchymal fibroelastosis (PPFE) have been identified in patients with compelling etiologic correlates commonly associated with the development of interstitial lung disease (ILD), including familial interstitial pneumonitis ; recurrent lower respiratory tract lung infections ; lung, bone marrow, and hematopoietic cell transplantation ; chemotherapeutic medications (drug-induced PPFE) ; occupational exposure to aluminosilicate dust or asbestosis ; and systemic autoimmune diseases, such as rheumatoid arthritis and scleroderma. As with the other IPs/ILDs, PPFE is described as idiopathic when no associated causative etiology can be identified. iPPFE was recently recognized as a distinct entity in the American Thoracic Society/European Respiratory Society 2013 update on the IIPs and was categorized as one of the two rare IIPs, along with lymphoid IP.